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pISSN: 1011-8942 eISSN: 2092-9382

2017 The Korean Ophthalmological SocietyThis is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses /by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

194

Original Article

Korean J Ophthalmol 2017;31(3):194-201ht tps: / /doi.org /10.3341/k jo.2016.0034

Orbital Lymphangioma: Characteristics and Treatment Outcomes of 12 Cases

Young Jun Woo1, Chang Yeom Kim1, Bradford Sgrignoli2, Jin Sook Yoon1

1Institute of Vision Research, Department of Ophthalmology, Yonsei University College of Medicine, Seoul, Korea2Department of Ophthalmology, Saint Johns Episcopal Hospital, Far Rockaway, NY, USA

Purpose: To report the patient characteristics and treatment outcomes in 12 cases of orbital lymphangioma.Methods: In this study, orbital lymphangioma was diagnosed based on clinical, radiologic (computed tomog-

raphy, magnetic resonance imaging), and histologic findings when possible. Patients whose vision was not

compromised by orbital lymphangioma, or that did not have increased intraocular pressure (IOP), received

oral corticosteroids. Orbital lymphangioma that affected vision or increased IOP was treated by surgery, which

included aspiration of blood or partial resection with or without injection of a sclerosant.

Results: Four patients without compromised vision responded well to oral corticosteroids. Eight patients with compromised vision underwent some form of surgery. Bleeding recurred in three patients after aspiration of

blood and in two after partial resection and intralesional injection of a sclerosant. Overall, five patients were

treated successfully by aspiration of blood, intralesional injection of a sclerosant, and application of continuous

negative pressure by appropriate drainage. Partial resection was successful in two patients with organized he-

matoma.

Conclusions: Orbital lymphangioma that does not compromise vision can be treated medically using oral corti-costeroids. Patients with threatened vision or elevated IOP due to acute hemorrhage should be treated by as-

piration of blood, intralesional injection of a sclerosant, and application of continuous negative pressure. Partial

resection may be effective only in patients with organized hematoma.

Key Words: Hemorrhage, Lymphangioma, Sclerotherapy, Treatment outcome

Lymphangioma is a benign tumor of the lymphatic sys- tem that is characterized by abnormal endothelial-lined channels [1]. Generally found in the head and neck region, these tumors constitute 0.3% to 4% of all orbital tumors and are not considered hamartomas because the orbit does not usually contain lymphatic vessels [2,3]. Some patients with orbital lymphangioma may develop proptosis, either slowly as the mass invades the orbit or suddenly during

Received: April 11, 2016 Accepted: May 18, 2016

Corresponding Author: Jin Sook Yoon, MD, PhD. Institute of Vision Research, Department of Ophthalmology, Yonsei University College of Medicine, #50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Korea. Tel: 82-2-2228-3570, Fax: 82-2-312-0541, E-mail: yoonjs@yuhs.ac

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YJ Woo, et al. Treatment of Orbital Lymphangioma

hemorrhage of a lesion [3]. In childhood, the diagnosis is often made when proptosis occurs after bleeding as a re-sult of minor trauma or upper respiratory infection, and may even occur spontaneously. The lymphangioma itself or the associated bleeding can restrict ocular motility and cause compressive optic neuropathy because of its mass [4].

Lymphangiomas can also infiltrate diffusely into sur-rounding vital structures such as the optic nerve. This char-acteristic, along with the associated hemorrhage, presents many surgical challenges and renders management of orbit-al lymphangiomas very difficult. Several methods have been used to treat orbital lymphangioma, including systemic cor-ticosteroids, injection of a sclerosant, and surgical excision, but currently, there are no definitive curative treatments [5,6]. Case reports and studies of orbital lymphangioma are rare in the Korean population. Here we describe the characteristics and treatment outcomes in 12 Korean patients with orbital lymphangioma.

Materials and Methods

We retrospectively reviewed the medical records of 12 patients that had been diagnosed with orbital lymphangio-ma between January 2005 and May 2015 at the Severance Hospital, Yonsei University College of Medicine, Seoul, Ko-rea. The diagnosis was based on clinical manifestations, imaging studies, including computed tomography and mag-netic resonance imaging (MRI), and histologic findings when possible. Ophthalmic evaluation included measure-ment of visual acuity, intraocular pressure (IOP), ocular motility, and Hertel exophthalmometry. Patients without elevated IOP and no immediate threat to vision were treat-ed with oral corticosteroids. Patients threatened with loss of vision or severe disfigurement underwent aspiration of blood or partial resection (with or without intralesional in-jection of a sclerosant and application of continuous nega-tive pressure). The study adhered to the tenets of the Dec-laration of Helsinki. Ethical approval to conduct the study was obtained from the institutional review board at the Sev-erance Hospital of Yonsei University (No. 4-2015-0342).

Results

The demographic and clinical characteristics of the pa-

tients are detailed in Table 1. The mean patient age at pre-sentation was 14.7 years (range, 1 to 76; median, 5.5). Seven patients were male and five were female. Ten patients were diagnosed with orbital lymphangioma in childhood. The mean follow-up period was 22.8 months (range, 2 to 68). All of the patients initially presented with proptosis due to lesional hemorrhage and mass effect of the tumor. The causes of bleeding included blunt ocular trauma (n = 3), upper respiratory infection (n = 2), and a spontaneous event (n = 7). The tumors were classified as superficial (n = 0), deep (n = 6), combined (n = 4), or complex (n = 2) [5].

In patients that could be tested for visual acuity (n = 10), all but one had a visual acuity at presentation above 20 / 50. One patient had a visual acuity of 10 / 200 and two pa-tients could not be tested because of their young age. During treatment, one patient showed worsening of visual acuity to light perception negative, but the other patients did not experience any further impairment of visual acui-ty. An IOP above 21 mmHg was recorded in four patients. All patients had limited ocular motility and three had compressive optic neuropathy before or during follow-up.

Four patients (cases 6, 8, 11, and 12) were treated with oral corticosteroids only (Fig. 1A and 1B), but eight re-quired some form of surgical intervention. Five of the eight surgically treated patients experienced a recurrence of one or more hemorrhagic episodes. Among these, two patients (cases 4 and 7) underwent a partial resection and received an intralesional injection of a sclerosant, and three patients (cases 1, 5, and 10) underwent aspiration of blood (Fig. 2). In the two patients who underwent partial resection (cases 4 and 7), bleeding recurred from posterior residual lymph-angioma tissue. At reoperation, this residual tissue was difficult to approach due to its deep location. Overall, five patients (cases 1, 2, 3, 4, and 7) were treated successfully by aspiration of blood, intralesional injection of a sclero-sant (OK-432 in two cases and bleomycin in three cases), and application of continuous negative pressure by appro-priate drainage. Two patients (cases 9 and 10) experienced organized hematoma that caused proptosis for several months, but after partial resection, these patients eventual-ly had favorable outcomes (Table 1).

Case 4 description

A 4-year-old girl presented with a 1-day history of left-sided proptosis and eyelid swelling (Fig. 3A). She had

196

Korean J Ophthalmol Vol.31, No.3, 2017

Tabl

e 1.

Dem

ogra

phic

cha

ract

erist

ics,

clin

ical

feat

ures

, and

trea

tmen

t mod

aliti

es in

pat

ient

s with

orb

ital l

ymph

angi

oma

Cas

eSe

xA

ge a

t pr

esen

tatio

n (y

r)FU

(m

on)

Initi

al

VAIn

itial

IOP

(mm

Hg)

Fina

l VA

Cau

se

of b

leed

ing

Cla

ssifi

catio

nTr

eatm

ent /

tim

e to

recu

rren

ceR

ecur

renc

e (n

)

1F

312

10 /

200

6420

/ 30

Blu

nt tr

aum

aC

ombi

ned

1st:

Blo

od e

vacu

atio

n / 2

wk

2nd:

Blo

od e

vacu

atio

n, O

K-4

32 in

ject

ion

/ 3 d

ay*

b

lood

eva

cuat

ion,

OK

-432

inje

ctio

n

1

2M

112

NA

56N

AB

lunt

trau

ma

Dee

pB

lood

eva

cuat

ion,

OK

-432

inje